Severe Skin Reactions: Clinical Picture, Epidemiology, Etiology, Pathogenesis and Therapy

Background: Severe skin reactions, mostly following medication use, are rare and can be associated with high mortality. A suitable treatment approach that is able to reduce mortality is needed. Methods: Recent publications on this topic were reviewed and evaluated. Results: In the case of the self-limiting diseases acute generalized exanthematous pustulosis (AGEP) and generalized bullous fixed drug eruption (GBFDE), there is no clear indication for systemic immunomodulating treatment, and supportive care remains the gold standard. The situation is less clear in the case of drug reaction with eosinophilia and systemic symptoms (DRESS); nevertheless, primarily in the case of severe organ involvement, systemic glucocorticosteroids are recommended. This is associated with complications and often also with virus reactivation, which may delay healing. The evidence on various immunomodulating therapies in Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) is controversial. Recent publications favor steroid pulse treatment, the tumor necrosis factor (TNF)-α inhibitor etanercept, as well as the calcineurin inhibitor cyclosporine A, with the latter representing the most promising approach. Conclusion: The rarity and unpredictability of the reactions make a randomized double-blind therapeutic trial extremely difficult. Using meta-analyses, it is possible to trace a trend in the use of systemic treatment options. Supportive care remains the most important treatment strategy for all clinical entities. © 2019, Springer Medizin Verlag GmbH, ein Teil von Springer Nature.